@article {117522, title = {Risk factors for pulmonary hypertension in patients with β thalassemia intermedia.}, journal = {European journal of internal medicine}, volume = {22}, year = {2011}, month = {2011 Dec}, pages = {607-10}, abstract = {

BACKGROUND: Pulmonary hypertension (PHT) is a common yet poorly understood complication of β thalassemia intermedia (TI).

METHODS: We herein evaluated risk factors for PHT in TI, through comparing 64 TI patients with evidence of PHT by symptomatology and echocardiography (Group I) to age- and sex-matched TI patients without PHT (Group II). Retrieved data included demographics, laboratory parameters, clinical characteristics, and received treatments that may influence PHT development; and reflected the period prior to PHT occurrence in Group I.

RESULTS: The mean age of Group I patients at development of PHT was 37.3{\textpm}10.6years; with 44\% being males. Among studied parameters, Group I patients were more likely to be splenectomized (4.9-times), transfusion-naive (3.5-times); hydroxyurea-naive (2.6-times), or iron chelation-naive (2.3-times); and have nucleated red blood cell count >=300{\texttimes}10(6)/l (2.59-times) or a previous history of thromboembolic events (3.69-times).

CONCLUSION: TI patients who eventually develop PHT may be identified early on by being splenectomized, having high nucleated red blood cell counts and a previous history of thromboembolism. Prospective clinical trials that evaluate the efficacy, safety, and cost effectiveness of transfusion, iron chelation, and hydroxyurea therapy in preventing PHT in TI are invited.

}, keywords = {Adult, beta-Thalassemia, Blood Coagulation, Erythrocyte Count, Female, Ferritins, Genotype, Humans, Hypertension, Pulmonary, Logistic Models, Male, Middle Aged, Multivariate Analysis, Retrospective Studies, Risk Factors, Splenectomy, Thromboembolism}, issn = {1879-0828}, doi = {10.1016/j.ejim.2011.05.013}, author = {Karimi, Mehran and Musallam, Khaled M and Maria Domenica Cappellini and Shahina Daar and Amal El-Beshlawy and Belhoul, Khawla and Saned, Mohamed-Salaheldin and Temraz, Sally and Koussa, Suzanne and Taher, Ali T} }